Acute Pain Due to Sickle-Shaped Cells

Article Reviewed: Stanley AC, Christian JM. Sickle Cell Disease and Periperative

Considerations: Review and Retrospective Report. J Oral Maxillofac Surg 2013;71(June):1027-1033.


Background: Sickle cell disease (SCD) occurs in the African-American population with an incidence of 1/500 births. Approximately 70.000 to 100.000 people in United States have SCD, and >2 million have sickle cell trait. Due to advances in medical screening tests, vaccines, and regular use of prophylactic antibiotics in the first few years of life, patients with SCD are livinglonger. There are 3 types of "normal"hemoglobin (a,a2,and fetal). Hemoglobin S (HbS) occurs when glutamic aci is substituted by valine at the sixth position on B-globin chain of hemoglobin. Normal red blood cells are round and resemble "biconcave discs" and often have a lifespan of 120 days. Changes in oxygen tension do not affect these normal erythrocytes. However, red blood cells with HbS distort in shape under low oxygen tension and resemble a rigid sickle shape. These distorted erythrocytes cannot flow throught capillary beds and blockage may occur, causing ischemia to various tissues and organs resulting in acute pain. The sickle-shaped red blood cells have a lifespan of only 10 to 12 days thus causing hemolytic anemia in patients.

Objective: To review SCD and discuss a retrospective study conducted at the Regional Medical Center in Memphis on patients who had SCD.

Design: Literature review and retrospective record evaluation.

Results: Twenty-nine patients were studied, and 21 procedures were done. IV fluids, type of anesthesia, thermoregulation, prophylactic antibiotics, and supplemantal oxygen were recorded on a table. Due to small sample size and an inconsistent protocol for patient management, a definitive conclusion could not be achieved. However, since no significant complications occurred in this retrospective study, it may be safe to treat SCD patients in an outpatient setting.

Conclusions: In SCD, a series of vasocclusive events occur resulting in end-organ necrosis, lung tissue destruction, and severe acute pain episodes. In severe cases of SCD, patients may have severe pain and acute chest syndrome. Other complications of SCD may include stroke, osteomyelitis, hematological disorders, acute splenic sequestration/splenomegaly, etc. Current medical treatmnet includes the drug hydroxyurea, which converts to nitric oxide when metabolized. It decreases pain crises and acute chest syndrome with minimal toxicity to tissues. Other therapies may include red blood cell transfusion or hematopoietic stem cell transplantation, but these treatments may have complications including graft rejection, infection, etc. Oral surgeons may need to perioperatively manage these patients with proper liquid hydration, supplemental oxygen, perfusion, thermoregulation, cautious sedation, antibiotics, and even blood transfusions if needed.

Reviewer's Comments: This authors of this article provided valuable insight on the management of patienets with SCD. Althought the sample size of patients included in this retrospective study was small, the table presented valuable data for further research and study on treatment of SCD patients in an outpatient setting with no complications.

Reviewer: Gargi Mukherji, DDS


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